ABSTRACT
Objective:To observe the clinical characteristics and optical coherence tomography (OCT) features of pseudopapilledema (PPE) combined with peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in children.Methods:A retrospective observational study. From October 2019 to May 2021, total 22 eyes from 12 children diagnosed as PPE combined with PHOMS in the Neuro-ophthalmology Department of The First Hospital of Xi’an (Affiliated of The First Hospital of Northwest University) were recruited. Among the children, 6 were male and 6 were female. The average age was (10.6±2.7) years. The average course from disease onset to diagnosis of PPE combined with PHOMS was (8.0±7.5) months. All patients underwent best corrected visual acuity (BCVA), relative afferent papillary defect (RAPD), Ishihara's test, fundus photography, OCT, fundus autofluorescence (FAF), ocular B-mode ultrasound, visual field and patternvisual evoked potential (P-VEP). The clinical and OCT characteristics of the patients were observed.Results:The anterior segments of the patients were normal. The intraocular pressures and Ishihara's test were all normal. All RAPD were negative. Total 22 eyes, BCVA was 1.0 in 21 eyes and one eye was 0.12. The fundus photography revealed blurred optic discs margin, showing mild to moderate edema-like elevation with more prominent in the nasal parts, presenting as a "C" shape halo. No obvious abnormal fluorescence was observed in FAF. The OCT scan of involvement eyes showed an elevated appearance in vary degrees, and the sharply marginated ovoid hyper-reflective mass-like structures which laterally herniated into the peripapillary region under retinal nerve fiber layer and above the Bruch membrane were detected with consecutive nasal enlargement scanning, corresponding to the nasal parts in the fundus photography. The higher degree of elevation, the larger the volume. Macular retina pigment epithelium layer and ganglion cell thickness were normal. Ocular B-mode ultrasound showed that the head of the optic nerve in the posterior wall of the eyeball (in front of the optic disc) was elevated in all affected eyes, and there was no strong signal echo in it. Visual field examination showed physical blind spot enlargement in 3 eyes and visual field defect in 2 eyes. P-VEP examination showed that the peak was slightly delayed in 3 eyes and the amplitude was slightly reduced in 3 eyes.Conclusions:Enlarged nasal optic disc OCT scan can improve the detection rate of PHOMS. PHOMS were detected bilaterally in the cases with binocular PPE while only in the effected eye in the cases of monocular PPE; the higher degree of PPE, the lager volume of PHOMS. PHOMS were could contribute to the diagnosis of PPE in children.
ABSTRACT
Abstract In children, optic disc drusen (ODD) are often mistaken for papilledema, this being the prin-cipal differential diagnosis. This report describes the case of an 11-year old patient with ODD, in which the condition was initially diagnosed as papilledema, and the patient referred for pulse therapy. Fundoscopic examination is important because it is the first examination conducted by the ophthalmologist that is capable of revealing some characteristics of ODD that will aid in the differentiation between this disease and papilledema. In cases of ODD, the optic disc presents blurred margins and elevation of the disc borders, with clearly defined vessels at the papilla border. The differential diagnosis of ODD in children is challenging and requires appropriate management and follow-up to avoid iatrogenesis.
Resumo As drusas de disco óptico (DDO) em crianças são frequentemente confundidas com papiledema, sendo este o principal diagnóstico diferencial. Este artigo relata o caso de uma paciente de 11 anos com DDO, no qual o quadro foi inicialmente diagnosticado como papiledema, e a paciente encaminhada para pulsoterapia. O exame fundoscópico é importante por ser o primeiro exame realizado pelo oftalmologista que é capaz de revelar algumas características das DDO que auxiliarão na diferenciação do papiledema. Nos casos de DDO, o disco óptico apresenta margens mal definidas e bordas elevadas, com vasos na margem da papila bem definidos. O diagnóstico diferencial das DDO em crianças é desafiador e requer conduta e seguimento adequados para evitar iatrogenias.
Subject(s)
Humans , Female , Child , Optic Disk Drusen/diagnostic imaging , Papilledema/diagnostic imaging , Ultrasonography , Diagnosis, DifferentialABSTRACT
Optic disc drusen (ODD) is the accumulations of calcified hyaline-like material within the substance of the optic nerve head. Optic disc drusen, especially if it is bilateral, may mimic the clinical presentation of papilledema. Usually retinal nerve fiber layer (RNFL) thinning can be present in ODD. In this report we present uncommon RNFL changes in a patient with bilateral ODD. A 17-year-old male was referred by another center with a diagnosis of optic disc edema. The patients visual acuity, the slit-lamp examination and the intraocular pressures were normal in both eyes. On fundus examination, there were irregularly elevated discs bilaterally and the optic nerves appear with hazy disk margins. He did not have visual field defects in automated perimetry. Bilateral ODD were identified and confirmed by B-scan ultrasonography and optical coherence tomography (OCT) demonstrated 4 clock hours of RNFL thickening. Optic disc drusen may be misdiagnosed as papilledema. Thus, clinical suspicion of ODD is important in order to diagnose papilledema and prevents unnecessary interventions. Although most of eyes with ODD have normal or thinner RNFL thickness, some of these eyes can have thicker RNFL thickness.
As drusas do disco óptico (DDO) são depósitos de material hialino calcificado dentro da substância da cabeça do nervo óptico. Drusas do disco óptico, especialmente se for bilateral, podem apresentar o quadro clínico de edema de papila. Usualmente o espessamento da camada de fibras nervosas da retina (RCFN) podem estar presentes em DDO. Neste relato apresentamos o caso de um homem com 17 anos de idade que foi encaminhado por um outro centro, com o diagnóstico de edema do disco óptico. A acuidade visual do paciente, o exame de lâmpada de fenda e a pressão intraocular foram normais em ambos os olhos. No exame de fundo de olho havia discos elevados de forma irregular bilateralmente e os nervos ópticos com margens de disco nebulosas. Ele não tinha defeitos do campo visual em perimetria computadorizada. Drusas do disco óptico (DDO) bilateral foram identificados e confirmados pela ultrassonografia Bscan e tomografia de coerência óptica (TCO) que demonstraram 4 horas de relógio de RCFN com espessamento. As drusas do disco óptico podem ser diagnosticadas como papiledema. Assim, a suspeita clínica de DDO é importante a fim de evitar intervenções desnecessárias. Embora a maioria dos olhos com DDO têm espessura normal ou thinner RCFN, alguns desses olhos podem ter camada mais grossa na RCFN.
Subject(s)
Humans , Male , Adolescent , Optic Disk Drusen/diagnosis , Nerve Fibers/pathology , Papilledema/diagnosis , Retina/pathologyABSTRACT
BACKGROUND: Optic disc drusen, which are calcified deposits that form anterior to the lamina cribrosa in the optic nerve, may mimic papilledema. CASE REPORT: We report herein three cases referred to us with suspicion of disc swelling and papilledema. Following ophthalmologic evaluation with B-scan ultrasound, red-free fundus photography, and computed tomography, the diagnosis of papilledema was excluded in all cases and optic disc drusen was diagnosed. CONCLUSIONS: Clinical suspicion of optic disc drusen in cases presenting with swelling of the optic nerve head is important in order to avoid unnecessary interventions and anxiety. The reported cases highlight the commonly encountered clinical presentations and the practical aspects of diagnosis and management of optic disc drusen.